Ovarian sex-cord stromal tumours and small cell tumours: Pathological, genetic and management aspects

Ovarian cancer in adolescents and young adults

“…FOXL2 mutations are rarely detected, but in one series, AKT1 mutations were observed in 63% cases . Although prognosis is generally better than for adult‐type GrCT, late‐stage disease is associated with an aggressive clinical course and long‐term survival is rare …”

“…In contrast, juvenile GrCTs are rare, comprising only 5% of all granulosa cell tumors, and nearly always occur in children (85% cases). Approximately 80% patients have precocious puberty at presentation .…”

“…In contrast, juvenile GrCTs are rare, comprising only 5% of all granulosa cell tumors, and nearly always occur in children (85% cases). Approximately 80% patients have precocious puberty at presentation . FOXL2 mutations are rarely detected, but in one series, AKT1 mutations were observed in 63% cases .…”

“…Adult‐type GrCTs have a median age of onset of 50 to 55 years and commonly present with signs of hyperestrogenism. FOXL2 has been identified as a driver gene and is useful in confirming the diagnosis .…”

“…SLCTs account for less than 0.5% ovarian SCSTs overall, but up to 20% in children . Lower patient age correlates with less well‐differentiated disease and a worse prognosis . SLCTs are associated with hereditary mutations in DICER1 (60%) .…”